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Livedoid vasculopathy
Other Resources UpToDate PubMed

Livedoid vasculopathy

Contributors: Amy E. Blum MA, Tyler Werbel MD, Patricia Ting MD, Ben Barankin MD, Eric Ingerowski MD, FAAP, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Livedoid vasculopathy (LV), also known as livedoid vasculitis, presents with retiform purpura or ulcers on the lower legs that may progress to atrophie blanche (AB). LV is caused by vaso-occlusion of the cutaneous microcirculation that manifests histopathologically as hyalinization of vessels leading to fibrin thrombi. LV is a rare condition, with an estimated incidence of 1:100 000 per year and an increased incidence during summer months and during pregnancy. It tends to affect young to middle-aged adults, with a median age of onset of 30-50 years, and has a female-to-male ratio of 2-3:1.

The pathogenesis of LV is not well understood, but it is likely related to increased coagulability and/or decreased fibrinolysis. The strongest risk factor is a hypercoagulable state with laboratory evaluation positive for a known cause of thrombophilia in 40%-50% of patients in retrospective cohort studies. The second most common risk factor is connective tissue disease. LV can also be associated with monocolonal gammopathy, with hepatitis B or C, or can be idiopathic.

LV follows a chronic time course, with lesions often developing for months before a definitive diagnosis is made. In a recent case series, the median interval between symptom onset and diagnosis was 10 months and between onset and treatment was 22.5 months.

The condition is rare in children.

Codes

ICD10CM:
L95.0 – Livedoid vasculitis

SNOMEDCT:
238762002 – Livedoid vasculitis

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Last Reviewed:06/03/2020
Last Updated:06/04/2020
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Livedoid vasculopathy
Livedoid vasculopathy : Lower leg, Painful skin lesions, Feet or toes
Clinical image of Livedoid vasculopathy
A close-up of porcelain white, depressed scars with adjacent crusting and prominent telangiectasia.
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